Anesth Pain Med.  2011 Jul;6(3):290-293.

Clinical manifestations and anesthetic management of Kearns-Sayre syndrome: A case report

Affiliations
  • 1Department of Anesthesiology and Pain Medicine, School of Medicine, Yeungnam University, Daegu, Korea. adjee@medical.yeungnam.ac.kr

Abstract

Kearns-Sayre syndrome (KSS) is a mitochondrial disorder resulting in multi-system dysfunction. A 14-year-old boy with KSS underwent external levator muscle resection for correction of ptosis. There were no abnormalities on the pre-operative evaluation, except for low-grade heart block and ocular problems. General anesthesia was conducted with a minimum dose of thiopental sodium and sevoflurane under close monitoring, and a laryngeal mask was inserted without muscle relaxation. The surgery was uneventful; however, a careful approach was required during anesthesia because life-threatening complications may occur in patients with KSS.

Keyword

General anesthesia; Kearns-Sayre syndrome; Mitochondrial disease

MeSH Terms

Adolescent
Anesthesia
Anesthesia, General
Heart Block
Humans
Kearns-Sayre Syndrome
Laryngeal Masks
Methyl Ethers
Mitochondrial Diseases
Muscle Relaxation
Muscles
Thiopental
Methyl Ethers
Thiopental
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